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Pediatric nephrology

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405 papers 100 to 500 followers
By P O Pediatrics, Nephrology
Agnes Trautmann, Beata S Lipska-Ziętkiewicz, Franz Schaefer
Background: Steroid resistant nephrotic syndrome (SRNS) is a rare condition, accounting for 10-15% of all children with idiopathic nephrotic syndrome. SRNS can be caused by genetic abnormalities or immune system dysfunction. The prognosis of SRNS varies from permanent remission to progression to end-stage kidney disease, and post-transplant recurrence is common. Objectives: The PodoNet registry project aims to explore the demographics and phenotypes of immune-mediated and genetic forms of childhood SRNS, to assess genotype-phenotype correlations, to evaluate clinical management and long-term outcomes, and to search for novel genetic entities and diagnostic and prognostic biomarkers in SRNS...
2018: Frontiers in Pediatrics
Valentin Amrhein, Sander Greenland, Blake McShane
No abstract text is available yet for this article.
March 2019: Nature
Adela Urisarri, Marta Gil, Natalia Mandiá, Luís Aldamiz-Echevarría, Roca Iria, Domingo González-Lamuño, María-Luz Couce
To evaluate the prognostic significance of factors frequently associated with a reduction in renal mass, such as prematurity, low birth weight, and congenital anomalies of kidney and urinary tract (CAKUT), in patients with solitary functioning kidney (SFK) and investigate signs of early renal injury due to glomerular hyperfiltration damage or dysplasia in the remaining kidney.Retrospective observational study of congenital SFK diagnosed and followed at a tertiary care hospital over a period of 10 years in which 32,900 newborns underwent routine neonatal abdominal ultrasound screening...
August 2018: Medicine (Baltimore)
Paolo Malvezzi, Thomas Jouve, Johan Noble, Lionel Rostaing
The number of kidney transplant candidates is increasing sharply. Among them, at least 20% are HLA sensitized. For these patients, in the setting of both living- and deceased-donor kidney transplant, we may face donor-specific alloantibodies at pretransplant. In such cases, the microlymphocytotoxicity crossmatch may or may not be positive. Kidney transplant with donor-specific antibodies at pretransplant is known as HLA-incompatible transplant. At present, we can use many methods to ensure that the kidney transplant is successful, provided that the recipient is desensitized before or after transplant...
August 2018: Experimental and Clinical Transplantation
Alexandra Cambier, Olivia Boyer, Georges Deschenes, James Gleeson, Anne Couderc, Julien Hogan, Thomas Robert
IgA nephropathy (IgAN) is one the most common primary glomerulonephritis in children and adolescents worldwide, with 20% of children developing end-stage kidney disease (ESKD) within 20 years of diagnosis. There is a need for treatment guidelines, especially for steroids in children with primary IgAN, since the STOP-IgA trial casts doubts on the use of steroids in adults with intermediate risk. Pediatricians are prone to prescribe steroids in addition to renin-angiotensin system blockade (RASB) when proteinuria is > 0...
February 18, 2019: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Mineaki Kitamura, Yoko Obata, Yuki Ota, Kumiko Muta, Hiroshi Yamashita, Takashi Harada, Hiroshi Mukae, Tomoya Nishino
Subepithelial deposits are observed in rare adult IgA nephropathy (IgAN) cases and are a key diagnostic finding in IgA-dominant infection-related glomerulonephritis (IgA-IRGN). Sometimes, it is difficult to distinguish IgA-IRGN from IgAN without a precise clinical history. We hypothesized that some IgA-IRGN cases might be diagnosed as IgAN with subepithelial deposits (IgAN-SD) and aimed to clarify the significance of subepithelial deposits in patients diagnosed with IgAN. We examined 464 patients diagnosed with IgAN at Nagasaki University Hospital and affiliated hospitals between 1996 and 2013...
2019: PloS One
Nicolas Maillard, Christophe Mariat
No abstract text is available yet for this article.
February 18, 2019: Nephrology, Dialysis, Transplantation
Sergio Camilo Lopez-Garcia, Francesco Emma, Stephen B Walsh, Marc Fila, Nakysa Hooman, Marcin Zaniew, Aurélia Bertholet-Thomas, Giacomo Colussi, Kathrin Burgmaier, Elena Levtchenko, Jyoti Sharma, Jyoti Singhal, Neveen A Soliman, Gema Ariceta, Biswanath Basu, Luisa Murer, Velibor Tasic, Alexey Tsygin, Stéphane Decramer, Helena Gil-Peña, Linda Koster-Kamphuis, Claudio La Scola, Jutta Gellermann, Martin Konrad, Marc Lilien, Telma Francisco, Despoina Tramma, Peter Trnka, Selçuk Yüksel, Maria Rosa Caruso, Milan Chromek, Zelal Ekinci, Giovanni Gambaro, Jameela A Kari, Jens König, Francesca Taroni, Julia Thumfart, Francesco Trepiccione, Louise Winding, Elke Wühl, Ayşe Ağbaş, Anna Belkevich, Rosa Vargas-Poussou, Anne Blanchard, Giovanni Conti, Olivia Boyer, Ismail Dursun, Ayşe Seda Pınarbaşı, Engin Melek, Marius Miglinas, Robert Novo, Andrew Mallett, Danko Milosevic, Maria Szczepanska, Sarah Wente, Hae Il Cheong, Rajiv Sinha, Zoran Gucev, Stephanie Dufek, Daniela Iancu, Robert Kleta, Franz Schaefer, Detlef Bockenhauer
BACKGROUND: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. METHODS: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form. RESULTS: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%)...
February 18, 2019: Nephrology, Dialysis, Transplantation
Tadasu Kojima, Go Hirose, Shuuhei Komatsu, Taito Oshima, Kentaro Sugisaki, Tomohiro Tomiyasu, Noriko Yoshikawa, Muneharu Yamada, Takashi Oda
BACKGROUND: Anti-glomerular basement membrane (GBM) glomerulonephritis does not usually coexist with another glomerulonephritis such as IgA nephropathy. We present a rare case having a combination of these two diseases, and furthermore, histological evaluation could be performed before and after the development of anti-GBM glomerulonephritis over a period of only10 months. CASE PRESENTATION: A 66-year-old woman was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years...
January 25, 2019: BMC Nephrology
Francesco Garzotto, Marta Zaccaria, Enrico Vidal, Zaccaria Ricci, Anna Lorenzin, Mauro Neri, Luisa Murer, Federico Nalesso, Alfredo Ruggeri, Claudio Ronco
OBJECTIVES: Renal replacement therapy in infants and small children is the treatment of choice for severe oligoanuric renal dysfunction, with an increasing consensus that early initiation might contribute to preventing acute kidney injury complications. Safer renal replacement therapy devices specifically designed for neonates may contribute to ameliorating outcomes and increasing chances of survival. One of the crucial factors to achieve an effective renal replacement therapy in small infants is adequate vascular access...
March 2019: Pediatric Critical Care Medicine
Richard J H Smith, Gerald B Appel, Anna M Blom, H Terence Cook, Vivette D D'Agati, Fadi Fakhouri, Véronique Fremeaux-Bacchi, Mihály Józsi, David Kavanagh, John D Lambris, Marina Noris, Matthew C Pickering, Giuseppe Remuzzi, Santiago Rodriguez de Córdoba, Sanjeev Sethi, Johan Van der Vlag, Peter F Zipfel, Carla M Nester
The C3 glomerulopathies are a group of rare kidney diseases characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent complement C3 deposition in kidney biopsy samples. The two major subgroups of C3 glomerulopathy - dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) - have overlapping clinical and pathological features suggestive of a disease continuum. Dysregulation of the complement alternative pathway is fundamental to the manifestations of C3 glomerulopathy, although terminal pathway dysregulation is also common...
January 28, 2019: Nature Reviews. Nephrology
Charlotte Gimpel, E Fred Avni, Luc Breysem, Kathrin Burgmaier, Anna Caroli, Metin Cetiner, Dieter Haffner, Erum A Hartung, Doris Franke, Jens König, Max C Liebau, Djalila Mekahli, Albert C M Ong, Lars Pape, Andrea Titieni, Roser Torra, Paul J D Winyard, Franz Schaefer
Kidney cysts can manifest as focal disease (simple and complex kidney cysts), affect a whole kidney (eg, multicystic dysplastic kidney or cystic dysplasia), or manifest as bilateral cystic disease (eg, autosomal recessive polycystic kidney disease [ARPKD] or autosomal dominant polycystic kidney disease [ADPKD]). In children, as opposed to adults, a larger proportion of kidney cysts are due to genetic diseases (eg, HNF1B nephropathy, various ciliopathies, and tuberous sclerosis complex), and fewer patients have simple cysts or acquired cystic kidney disease...
December 18, 2018: Radiology
Marina Vivarelli, Francesco Emma
In the current issue of Kidney International, Sinha et al. report results of a randomized controlled trial comparing levamisole and mycophenolate mofetil for the second-line treatment of children with frequent relapses of nephrotic syndrome. This study suggests that these two agents may both be safe and effective. The present commentary addresses strengths and limitations of this important study.
January 2019: Kidney International
Hae-Yoon Grace Choung, Andrew S Bomback, M Barry Stokes, Dominick Santoriello, Eric S Campenot, Ibrahim Batal, Glen S Markowitz, Vivette D D'Agati
Morbid obesity, defined as body mass index (BMI) ≥40 kg/m2 , affects approximately 8% of United States adults and is a recognized risk factor for chronic kidney disease (CKD). We present the first focused biopsy-based study exploring the range of kidney diseases in this population. Among 3263 native kidney biopsies interpreted at Columbia University in 2017, we identified 248 biopsies from morbidly obese patients. In this cohort with median age of 53.5 years, 56% were female and median BMI was 44.0 kg/m2 ...
January 26, 2019: Kidney International
Brad H Rovin, Dawn J Caster, Daniel C Cattran, Keisha L Gibson, Jonathan J Hogan, Marcus J Moeller, Dario Roccatello, Michael Cheung, David C Wheeler, Wolfgang C Winkelmayer, Jürgen Floege
In November 2017, the Kidney Disease: Improving Global Outcomes (KDIGO) initiative brought a diverse panel of experts in glomerular diseases together to discuss the 2012 KDIGO glomerulonephritis guideline in the context of new developments and insights that had occurred over the years since its publication. During this KDIGO Controversies Conference on Glomerular Diseases, the group examined data on disease pathogenesis, biomarkers, and treatments to identify areas of consensus and areas of controversy. This report summarizes the discussions on primary podocytopathies, lupus nephritis, anti-neutrophil cytoplasmic antibody-associated nephritis, complement-mediated kidney diseases, and monoclonal gammopathies of renal significance...
February 2019: Kidney International
Michael Joannidis, Sebastian J Klein, Marlies Ostermann
No abstract text is available yet for this article.
January 14, 2019: Intensive Care Medicine
Judith Isabel Schimpf, Till Klein, Christina Fitzner, Frank Eitner, Stefan Porubsky, Ralf-Dieter Hilgers, Jürgen Floege, Hermann-Josef Groene, Thomas Rauen
BACKGROUND: The Oxford classification of IgA nephropathy (IgAN) defines histologic criteria (MEST-C) that provide prognostic information based on the kidney biopsy. There are few data on the predictive impact of this classification in randomized clinical trial settings. METHODS: We performed an exploratory analysis of MEST-C scores in 70 available renal biopsies from 162 randomized STOP-IgAN trial participants and correlated the results with clinical outcomes. Analyses were performed by researchers blinded to the clinical outcome of the patients...
November 19, 2018: BMC Nephrology
Corey Cavanaugh, Mark A Perazella
Automated urine technology and centralized laboratory testing are becoming the standard for providing urinalysis data to clinicians, including nephrologists. This trend has had the unintended consequence of making examination of urine sediment by nephrologists a relatively rare event. In addition, the nephrology community appears to have lost interest in and forgotten the utility of provider-performed urine microscopy. However, it is critical to remember that urine sediment examination remains a time-honored test that provides a wealth of information about the patient's underlying kidney disease...
September 21, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Grégory Jacquillet, Robert J Unwin
Inorganic phosphate (Pi) is an abundant element in the body and is essential for a wide variety of key biological processes. It plays an essential role in cellular energy metabolism and cell signalling, e.g. adenosine and guanosine triphosphates (ATP, GTP), and in the composition of phospholipid membranes and bone, and is an integral part of DNA and RNA. It is an important buffer in blood and urine and contributes to normal acid-base balance. Given its widespread role in almost every molecular and cellular function, changes in serum Pi levels and balance can have important and untoward effects...
November 5, 2018: Pflügers Archiv: European Journal of Physiology
Gastón J Piñeiro, Erika De Sousa-Amorim, Manel Solé, José Ríos, Miguel Lozano, Frederic Cofán, Pedro Ventura-Aguiar, David Cucchiari, Ignacio Revuelta, Joan Cid, Eduard Palou, Josep M Campistol, Federico Oppenheimer, Jordi Rovira, Fritz Diekmann
BACKGROUND: Chronic active antibody-mediated rejection (c-aABMR) is an important cause of allograft failure and graft loss in long-term kidney transplants. METHODS: To determine the efficacy and safety of combined therapy with rituximab, plasma exchange (PE) and intravenous immunoglobulins (IVIG), a cohort of patients with transplant glomerulopathy (TG) that met criteria of active cABMR, according to BANFF'17 classification, was identified. RESULTS: We identified 62 patients with active c-aABMR and TG (cg ≥ 1)...
October 11, 2018: BMC Nephrology
2018-11-05 21:56:30
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